Factor VII deficiency is characterized by epistaxis, bruising, hemarthrosis, menorrhagia, gastrointestinal bleeding, hematuria, and intracranial hemorrhage during infancy. Causes of acquired factor VII deficiency include liver disease, Vitamin K deficiency, and warfarin administration. Congenital factor VII deficiency is an autosomal recessive disorder, with the homozygotes having a severe deficiency and the heterozygotes a moderate deficiency of factor VII. Orthopedic, gynecological, cardiothoracic, and abdominal surgical procedures have been successfully performed in patients with factor VII deficiency both with and without factor VII replacement. We present two patients with moderate and moderately severe factor VII deficiency who successfully underwent intracranial procedures using plasma during the perioperative period for factor VII replacement. One patient successfully underwent stereotactic placement of mesial temporal lobe depth electrodes and subdural strip electrodes followed by anterior temporal lobectomy for medically refractory seizures. The second patient successfully underwent craniotomy for an olfactory groove meningioma. No bleeding complications were encountered with any of the three intracranial procedures performed. These cases represent the first reported cases of successful intracranial procedures in patients with factor VII deficiency, other than shunting procedures performed for intraventricular hemorrhage during infancy.